Pierre Robin sequence
Pronunciation: pee-YAIR ro-BAHN
Congenital condition involving micrognathia, glossoptosis, and cleft palate requiring staged surgical reconstruction.
Full Definition
Pierre Robin sequence is a constellation of congenital anomalies characterized by micrognathia (small jaw), glossoptosis (posterior displacement of the tongue), and often cleft palate. The condition results from mandibular hypoplasia during fetal development, leading to tongue displacement that interferes with palatal closure. Affected infants typically experience breathing and feeding difficulties due to airway obstruction. Treatment involves multidisciplinary care with staged surgical interventions, including possible mandibular distraction osteogenesis, tongue-lip adhesion, and cleft palate repair. The timing and sequence of procedures are critical for optimizing both functional outcomes and normal facial growth patterns.
Usage
Usage note: Use 'sequence' rather than 'syndrome' as it describes a cascade of developmental events rather than a genetic syndrome. Proper capitalization of the eponym is essential.
In Context
- "The newborn with Pierre Robin sequence required immediate airway management and feeding support." — Clinical assessment
- "Mandibular distraction osteogenesis was planned as the first stage in this Pierre Robin sequence case." — Surgical planning notes